1. PFF Teal = 0+160+175 MAIN COLORS PFF Green = 120+162+47 Light Green = 193+216+47 Red = 242+102+73 HIGHLIGHT COLORS Light Grey = 220+220+210 Dark Grey = 100+98+76 Black = 0+0+0 SYMPTOM MANAGEMENT MARYL E. KREIDER, MD, MSCE PULMONARY FIBROSIS MANAGEMENT STRATEGIES II: PLANNING FOR THE FUTURE NOVEMBER 14, 2015

2. 2 Questions to cover today  What are the most common symptoms?  Why do patients have them?  What can be done about them?  What other symptoms should be reported to the doctor?  Will any of these new fibrosis medications help with any of these symptoms?

3. 3 Most common symptoms  Shortness of breath (dyspnea) – 90%  Cough – 73-86%  Anxiety/depression 23-27%  Fatigue – 40-45%  Social isolation not reported in studies but a significant complaint of many of my patients

4. 4 Shortness of breath  Dyspnea is doctor speak for shortness of breath  Why are people with pulmonary fibrosis short of breath? “Road block” for gas exchange Changes in lung structure that affects how easily air moves in and out and where the air goes  Why am a mostly winded with exertion and not at rest?

5. 5 Shortness of breath  Things that may help Oxygen Pulmonary rehabilitation Narcotics Sildenafil (?) –probably only in a small group of patients will it really help

6. 6 Cough  PF patients cough for all the reasons everyone else coughs but much more  Things that may help Studied (though small studies) –Low dose steroids –Thalidomide Not studied but may make sense depending on your circumstances –Cough suppressants –Inhaled steroids –Anti post nasal drip medications –Anti-reflux medications –Aerosolized medications that open airways –Aerosolized salt water –Aerosolized numbing medications –Anti-nerve medications

7. 7 Sadness and Anxiety  While we know that both are very common in PF we don’t exactly know why Changes in oxygen levels change brain chemicals? Constant threat means always wound up? (Tiger analogy) Serious diagnosis that leads to big changes in what you can do and how you live  No studies to suggest how best treated Borrowing from other chronic lung diseases –Talk therapy –Medication

8. 8 Fatigue  Lots of potential contributing factors Low oxygen levels Higher frequency of sleep apnea and other types of sleep disordered breathing Being out of shape Being over weight  Ways to improve Wear oxygen to prevent desaturation –Exertion –Sleep –Repeat testing periodically to make sure flow needed isn’t changing If snore and sleepy get screened for sleep apnea and get it treated Pulmonary rehabilitation Weight loss

9. 9 Social Isolation Cough – hates the glares because they might be spreading something Shortness of breath – needs to walk slower and rest often so get left behind Oxygen – hard to carry and runs out after a period so must plan ahead Anxiety/Depression/Hopelessness Lose desire and become fearful to do anything Patient in a very small world

10. 10 Fighting Social Isolation  Opening the world back up Symptoms –Depression/anxiety –Cough –Shortness of breath Improving access to the world –Oxygen delivery –Pulmonary Rehabilitation –Scooters/wheelchairs/chair lifts Support –Patient advocacy groups –Access to new info/drugs/etc –Support groups

11. 11 Other symptoms to report  Most of these are due to the company that pulmonary fibrosis can keep rather than the disease itself Heartburn and/or difficulty swallowing Chest pain Swelling in the legs Passing out or nearly passing out Fever Changes in sputum  Or some can point to a different diagnosis Rash New joint pains Dry eyes or dry mouth  So its best to report just about anything

12. 12 What about these new fibrosis meds?  Studies that got Nintedanib and Pirfenidone approved used lung function tests to determine if they work – not symptoms  For Pirfenidone there is a theoretical reason to think it may help with cough In guinea pigs giving them pirfenidone made it harder to make them cough (Okazaki et al, Pulm Pharmacol Ther, 2013)  One center in Netherlands reported that of their patients on Pirfenidone 11/19 reported an improvement in cough after 1 month while 7/19 stayed the same and 1/19 got worse  Interesting but neither prove anything  Remember – both drugs are really intended to slow progression and should not really be expected to make you feel better  These drugs are also not appropriate for everyone with pulmonary fibrosis

13. 13 Symptom Management - Conclusions  What are the most common symptoms? Shortness of breath, cough, anxiety/depression, social isolation  Why do patients have them? “road block” and changes in geometry lead to low oxygen levels and increased work of breathing and increased cough sensitivity  What can be done about them? Oxygen, rehab, cough medications, sleep studies and therapy, etc.  What other symptoms should be reported to the doctor? Almost any because of the company fibrosis keeps and the possibility of other diagnoses  Will any of these new fibrosis medications help with any of these symptoms? Probably not – their goal is stabilization of lung function not symptom improvement

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